Cystic Fibrosis Drug Combo May Be Less Effective Than Hoped

One medication seems to partly counteract the other, suggests study on human cells
Source: Pulmonary Medicine News - Doctors Lounge - Category: Respiratory Medicine Authors: Tags: Pharmacy, Pulmonology, Research, News, Source Type: news

Related Links:

Authors: Brennan GT, Saif MW Abstract Pancreatic enzyme replacement therapy is safe and effective at treating pancreatic exocrine insufficiency. There are multiple causes of pancreatic exocrine insufficiency including chronic pancreatitis, cystic fibrosis and pancreatic cancer. Testing fecal elastase-1 level is useful for the diagnosis of pancreatic exocrine insufficiency. Starting doses of pancreatic enzyme replacement therapy should be at least 30-40,000 IU with each meal and 15-20,000 IU with snacks. pancreatic enzyme replacement therapy should be taken in divided doses throughout meals. Patients who do not resp...
Source: Journal of the Pancreas - Category: Gastroenterology Tags: JOP Source Type: research
Dietary intervention in cystic fibrosis (CF) has historically focused on high-energy diets to address malnutrition, with little attention on diet quality. With increased survival, CF complications such as impaired glucose tolerance (IGT) and cystic fibrosis related diabetes (CFRD) have increased in prevalence. In the absence of consensus on the management of IGT, the role of dietary intake, specifically carbohydrate quality, requires consideration.
Source: Clinical Nutrition - Category: Nutrition Authors: Tags: Original article Source Type: research
Conclusions: The pattern of airway colonisation in the Indian setting is different from the Caucasian population, and P. aeruginosa and Burkholderia cepacia complex appear early. Colonisation with P. aeruginosa benefits from therapy. In case of infection, care must be taken while initiating empiric therapy. It should be based on local antibiograms to prevent the emergence of resistant microbes.
Source: Indian Journal of Medical Microbiology - Category: Microbiology Authors: Source Type: research
Condition:   Cystic Fibrosis Interventions:   Dietary Supplement: Resveratrol;   Dietary Supplement: NR;   Dietary Supplement: Placebo Sponsor:   Virginia Commonwealth University Not yet recruiting
Source: - Category: Research Source Type: clinical trials
AbstractPurposeCystic fibrosis (CF) is a multisystem genetic disease caused by dysfunction of the epithelial anionic channel Cystic Fibrosis Transmembrane conductance Regulator (CFTR). Decreased mucociliary clearance because of thickened mucus is part of the pulmonary disease pathophysiology. It is controversial if the thickened  airway surface liquid (ASL) is caused by the deficient chloride secretion and excessive sodium (through ENaC) and water hyperabsorption from the periciliar fluid or by the lack of bicarbonate secretion with relative acidification of the ASL. Correlations between the magnitude of in vivo chlor...
Source: Lung - Category: Respiratory Medicine Source Type: research
Publication date: Available online 15 November 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Claudio D'Amore, Christian Borgo, Valentina Bosello-Travain, Jordi Vilardell, Valentina Salizzato, Lorenzo A. Pinna, Andrea Venerando, Mauro SalviAbstractF508del-CFTR, the most common mutation in cystic fibrosis (CF) patients, impairs CFTR trafficking to plasma membrane leading to its premature proteasomal degradation. Several post-translational modifications have been identified on CFTR with multiple roles in stability, localization and channel function, and the possibility to control the e...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - Category: Molecular Biology Source Type: research
Cystic fibrosis (CF) results from deficient CF transmembrane conductance regulator (CFTR) protein activity leading to defective epithelial ion transport. Pulmonary degradation due to excessive inflammation is the main cause of morbidity and mortality in CF patients. By analysing miRNAs (small RNAseq) in human primary air-liquid interface cell cultures, we measured the overexpression of miR-636 in CF patients compared to non-CF controls. We validated these results in explant biopsies and determined that the mechanism underlying miR-636 overexpression is linked to inflammation. To identify specific targets, we used bioinform...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Cystic fibrosis (CF) is characterised by a failure of mucociliary clearance and accumulation of viscid secretions in the airway, leading to recurrent infection. To mitigate this process, CF treatment guidelines recommend the regular use of an airway clearance technique (ACT), with exercise as an adjunct [1]. In people with CF (pwCF), exercise such as running or cycling, increases ease of sputum expectoration, minute ventilation and respiratory flow [2]. A comparison of one ACT with treadmill exercise showed that these approaches performed similarly over a single session [3].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Short Communication Source Type: research
Abstract Pathogenic variants in the Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR) are responsible for cystic fibrosis (CF), the commonest monogenic autosomal recessive disease, and CFTR-related disorders in infants and youth. Diagnosis of such diseases relies on clinical, functional, and molecular studies. To date, over 2,000 variants have been described on CFTR (~40% missense). Since few of them have confirmed pathogenicity, in silico analysis could help molecular diagnosis and genetic counseling. Here, the pathogenicity of 779 CFTR missense variants was predicted by consensus predictor PredictSNP and co...
Source: Genetics and Molecular Biology - Category: Genetics & Stem Cells Source Type: research
A new treatment for lung disease in cystic fibrosis (CF) for which there remains no cure could potentially benefit all patients, according to a University of Bristol study published in Chemical Science. The findings are an important step towards a new therapy addressing the fundamental cause of cystic fibrosis.
Source: University of Bristol news - Category: Universities & Medical Training Tags: Health, Research; Faculty of Life Sciences, Faculty of Life Sciences, School of Physiology, Pharmacology and Neuroscience, Faculty of Science, Faculty of Science, School of Chemistry; Press Release Source Type: news
More News: Cystic Fibrosis | Respiratory Medicine | Study